Cystic cyst

Cystic fibrosis

Cystic fibrosis (cystic fibrosis of the pancreas) is a severe hereditary disease characterized by damage to the exocrine glands and increased viscosity of secretions. This leads to damage to the lungs, pancreas, intestines, and less commonly, the liver and kidneys.

The cause of the disease is a mutation in the gene encoding a protein that regulates the transmembrane transport of chlorine ions. This disrupts the secretion of chlorides into the gland ducts, which leads to dehydration of the secretion and its increased viscosity.

Main clinical manifestations:

1. Pulmonary: chronic cough, bronchitis, pneumonia, bronchiectasis, respiratory failure.

2. Intestinal: malabsorption, fatty stools, abdominal pain, malnutrition.

3. Liver damage: cirrhosis.

4. The sweat test is positive - increased content of chlorides in sweat.

Diagnosis is based on the clinic, medical history, and sweat test. DNA diagnostics allows you to identify a mutation.

Treatment: antibiotics, mucolytics, pancreatic enzymes, vitamins, diet. The prognosis is serious, mortality reaches 50%. Early diagnosis and prevention of complications are important.